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Systemic lupus erythematosus During pregnancy

Systemic lupus erythematosus
During pregnancy

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Systemic lupus erythematosus During pregnancy

  1. 1. Systemic lupus erythematosus During pregnancy Aboubakr Elnashar Benha university, Egypt
  2. 2. Incidence Women > men (ratio 9:1) During the child-bearing years (ratio 15:1). Incidence: 1 in 1000 women Aboubakr Elnashar
  3. 3. Clinical features Systemic connective tissue disease Heterogeneous: variety of clinical& antibody patterns. Other autoimmune disorders: 6%. Periods of disease activity (flares)& remissions. Average age at diagnosis: 30 y Aboubakr Elnashar
  4. 4. Commonest clinical feature (90%): Arthritis: non­erosive, peripheral, tenderness& swelling. Other features: Skin: (80%): malar rash Photosensitivity vasculitic lesions on the "fingertips& nail folds, Raynaud's phenomenon Discoid lupus. Aboubakr Elnashar
  5. 5. Serositis: pleuritis, pericarditis Renal: glomeru­lonephritis with proteinuria& cellular casts Neurological: psychosis, seizures or chorea. Haematological: haemolytic anaemia, thrombocytopenia & lymphopenia or leukopenia. Aboubakr Elnashar
  6. 6. Pathogenesis Cause: Un known Genetic predisposition Environmental triggers e.g. ultraviolet light or viral infection. Immunological: polyclonal B-cell activation impaired T-cell regulation of the immune response failure to remove immune complexes. Circulating non-organ-specific autoantibodies. Deposition of immune complexes: vasculitis. Aboubakr Elnashar
  7. 7. Diagnosis American Rheumatic Association criteria Many patients have a lupus-like illness without fulfilling these. 1. CBC: Normochromic normocytic anaemia Neutropenia Thrombocytopenia. 2. ESR: raised {high immunoglobulin levels} 3. CRP: Normal 4. 3rd or 4th components of complement: low or falling: active disease. Aboubakr Elnashar
  8. 8. 5. Auto-antibody a. The most common: Antinuclear antibody (ANA): (96%) Titres do not change with disease activity. b. The most specific antibodies to double-stranded DNA (78%) and Smith (Sm). Glomerulonephritis occurs more frequently in women with these antibodies. c. Other anti-Ro and anti-La: (30%) anticardiolipin antibodies (aPLs): (40%). Aboubakr Elnashar
  9. 9. Effect of pregnancy on SLE Flares: Increases: from 40% to 60%. When: at any stage of pregnancy or the puerperium more likely immediately postpartum: little evidence Prediction: not possible more likely if disease has been active within 6 ms of conception. Aboubakr Elnashar
  10. 10. Diagnosis during pregnancy: difficult {many features such as hair loss, oedema, palmar & facial erythema, fatigue, anaemia, raised ESR & musculoskeletal pain also occur in normal pregnancy}. most commonly involving the skin& joints Prevention: prophylactic steroids or routine increases of dose: no effect & not recommended Aboubakr Elnashar
  11. 11. Women with lupus nephritis SLE nephropathy may manifest for the first time in pregnancy. Risk of deterioration Moderate renal impairment (serum creatinine 125- 200 umol/I): uncomplicated pregnancies. High baseline serum creatinine: Inc risk of deterioration Aboubakr Elnashar
  12. 12. Preconception counseling. 1. Delay pregnancy until at least 6 ms after a lupus nephritis flare. 2. Prediction of the risks to the woman& fetus: anti-Ro/La aPLs Renal function Blood pressure Aboubakr Elnashar
  13. 13. Effect of SLE on pregnancy 1. The increased risks  spontaneous miscarriage fetal death pre-eclampsia preterm delivery IUGR  Related to 1. Age, parity 2. anticardiolipin antibodies 3. lupus anticoagulant 4. lupus nephritis 5. hypertension 6. active disease at the time of conception or 7. first presentation of SLE during pregnancy.Aboubakr Elnashar
  14. 14. During pregnancy, lupus improves in a third of women remains unchanged in a third worsens in the remaining third. Clinical condition can worsen or flare without warning (Khamashta and colleagues, 1997). Risk of major morbidity during pregnancy 7% (Petri, 1998) Lupus can be life threatening to both the mother and her fetus-infant. Aboubakr Elnashar
  15. 15. Good pregnancy outcome if: 1. Lupus activity has been quiescent for at least 6 months before conception 2. No active renal involvement manifest by proteinuria or renal dysfunction 3. Superimposed preeclampsia does not develop 4. No evidence of antiphospholipid antibody activity. Aboubakr Elnashar
  16. 16. Renal lupus: increased risk of fetal loss, PET, IUGR particularly if there is hypertension or proteinuria. Women in remission, but without hypertension, renal involvement or aPLs: Risk of pregnancy loss& PET is not higher than in the general population. 2. Chorea very rare complication of pregnancy in women with SLE or aPLs. Aboubakr Elnashar
  17. 17. Management Preconception counseling 1. Prediction of the risks to the woman& fetus:  anti-Ro/La  aPLs  Renal  Blood pressure. 2. Outcome is improved if conception occurs during disease remission. Aboubakr Elnashar
  18. 18. Multidisciplinary team in combined clinics: Physicians& obstetricians Monitor 1. disease activity 2. Fetal growth 3. uterine artery Doppler blood flow at 20-24 w 4. umbilical artery blood flow from 24 w Aboubakr Elnashar
  19. 19. Baseline values in early pregnancy: 1. FBC 2. KFT: U, creatinine, uric acid, quantify any proteinuria. 3. LFT: 4. anti DNA 5. Complement titres 5. Electrolytes Serial measurements at intervals dependent on disease severity Aboubakr Elnashar
  20. 20. Disease flare Symptoms: Arthralgia, pleuritic pain, skin rash  Lab: A. Urine: Red blood cells or cellular casts B. Rising anti-DNA antibody titre C. Fall in complement levels >25% fall in C3 or C4 suggests active SLE. N.B. elevation of complement split products, particularly Ba & Bb, often accompanies flares, so high ratios of CH50: Ba may differentiate PET from active lupus. Aboubakr Elnashar
  21. 21. Treatment Corticosteroids are the drugs of choice. Aboubakr Elnashar
  22. 22. Hydroxychloroquine (Plaquenil) should be continued {stopping may precipitate flare}. For control of hypertension: Drug of choice: methyldopa 2nd -line agents: nifedipine or hydralazine Although long-term hydralazine& methyldopa use may rarely induce a SLE-like syndrome, they are not contraindicated in SLE. Aboubakr Elnashar
  23. 23. Differentiation of active renal lupus from PET Difficult 1. The two conditions may be superimposed. 2. Hypertension, proteinuria, thrombocytopenia& even renal impairment are all features of PET 3. A doubling of baseline proteinuria may be expected in pregnancy but more than this would be indicative of either worsening lupus nephritis or PET} Aboubakr Elnashar
  24. 24. How: 1. Hyperuricaemia & abnormal LFT point more towards PET. 2. Renal biopsy The only definitive investigation Rarely undertaken in pregnancy. More likely to be appropriate prior to fetal viability Aboubakr Elnashar
  25. 25. TT of active lupus nephritis Increase oral prednisolone Pulsed IV methyl predisolone Azathioprine (Imuran). Cyclophos­phamide: Rarely used Aboubakr Elnashar
  26. 26. If lupus flare & PET cannot be dd beyond 24-28 w, when the fetus is viable: Delivery {cure PET allow administration of cyclophosphamide} Aboubakr Elnashar
  27. 27. Conclusions There is an increased rate of flare during pregnancy. Disease flares must be actively managed with corticosteroids. Adverse pregnancy outcome is related to the presence of renal involvement, hypertension, antiphospholipid antibodies& disease activity at the time of conception. These factors increase the risks of spontaneous miscarriage, fetal death, PET, PTL& IUGR. Aboubakr Elnashar
  28. 28. Pregnancy care is best undertaken in combined clinics allowing close monitoring of disease activity, fetal growth& well-being. In Ro-positive mothers risk of transient neonatal cutaneous lupus: 5% risk of CHB: 2%. Aboubakr Elnashar
  29. 29. Aboubakr Elnashar Thanks